38
Omphalocele/exomphalos
Thomas R Weber
History
Ambrose Paré, the famous French surgeon, first described
an infant with omphalocele in 1634. Although small
omphaloceles were subsequently successfully repaired,
there were few reported survivors of larger abdominal
wall defects until the 1940s, when Gross described a
two-stage closure of an omphalocele using skin flaps
followed by ventral hernia repair. Further advancement
in the treatment of massive omphalocele defects occurred
when Schuster devised an extracelomic ‘pouch’ to house
eviscerated bowel temporarily. This was later modified by
Allen and Wrenn, who devised the additional innovation
of staged reduction of abdominal contents to allow gradual
enlargement of the abdominal cavity. The development of
total parenteral nutrition in the 1960s allowed vigorous
nutritional support of infants with abdominal wall defects,
in whom a period of 1–3 weeks of intestinal dysfunction
is expected after the operation. The basic principles of
occasional non-operative therapy, primary closure when
possible, and staged reduction with a temporary Silastic
‘pouch’, remain the mainstays of contemporary therapy
for these congenital defects.
Embryology
Although controversy continues regarding the
similarities, relationships, and embryological events
surrounding omphalocele and gastroschisis, it is probably
most reasonable to consider these as separate entities.
Omphalocele is basically a persistence of the body stalk
in the midline, where somatopleure normally develops.
Failure of return of the normally herniated midgut at
fetal week 12 either causes or aggravates the condition.
Unless ruptured, omphaloceles are covered with a sac
consisting of inner peritoneum and outer amnion. Infants
with omphalocele frequently have associated anomalies
(cardiac, renal), chromosome abnormalities (trisomy
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Chapter: 38
13–15, 16–18), or recognizable syndrome associations
(Beckwith–Wiedemann, Cantrell’s pentalogy, caudal
regression).
Principles and justification
Because of the possibility of serious, life-threatening, or
even lethal associated anomalies, infants with omphalocele
are occasionally treated non-operatively. Painting the sac
with agents designed to induce eschar formation, followed
by gradual epithelialization from the base of the defect
upwards, will eventually produce a covered ventral hernia.
Early use of alcohol, iodine, and mercury-containing
compounds produced toxicity due to systemic absorption
of these compounds, and they have therefore been largely
replaced by silver nitrate solutions or silver sulfadiazine
cream.
The operative treatment of choice for small- to medium-
sized omphaloceles is excision of the sac, with primary
closure of fascia and skin. If fascia closure increases
intra-abdominal pressure sufficiently to cause respiratory
embarrassment, skin closure alone, with later repair of
the ventral hernia, is advisable. For giant omphaloceles,
frequently containing liver as well as bowel, attaching a
Silastic ‘pouch’ to the fascia allows gradual (10–14 days)
reduction of the contents into the abdominal cavity, with
eventual skin flap closure. Occasionally, a prosthetic patch
is needed to close the fascial defect in this setting, but skin
must be mobilized sufficiently to cover the prosthesis.
Preoperative assessment and
preparation
Newborn infants with omphalocele must be placed
immediately in a warm, aseptic environment to prevent
evaporative fluid loss, hypothermia, and infection. A non-
adherent sterile gauze can be placed on the defect, covered
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by transparent plastic wrap. Alternatively, a transparent
plastic drawstring ‘bowel bag’ may be used, which can be
kept sterile in the delivery room. The lower two-thirds (to
the axillae) of the neonate can be placed within the bag.
Safe transport of the infant to a center that is experienced
in the management of these complex infants can then be
accomplished.
Infants with large omphaloceles, especially when the
liver is in the sac, should be positioned on their side to
prevent twisting of the inferior vena cava from the sac
‘tipping’ to one side. Alternatively, rolls can be used to
support the sac if the infant is placed supine.
Intravenous access must be established soon after birth
to replace evaporative fluid loss and administer broad-
spectrum antibiotics. Placement of the intravenous line
above the diaphragm is preferable because of the possibility
of inferior cava compression and partial obstruction as
the eviscerated bowel and/or liver are reduced. An oral
Operations
Small- to moderate-sized omphalocele
1 A small (‘hernia into cord’) or moderate omphalocele,
which may contain a small portion of liver, has the
umbilical cord inserted into the top of the sac.
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Operations  301
gastric or nasogastric tube should be placed to prevent
gastric distension. Preoperative endotracheal intubation
is reserved for premature infants or those with significant
respiratory distress. The latter is occasionally encountered
because pulmonary hypoplasia can be associated with
omphalocele. All infants with omphalocele should undergo
complete cardiac and renal evaluation before they are
subjected to operative repair.
Anesthesia
General, endotracheal anesthesia, with complete muscle
paralysis, is recommended for all infants with omphalocele.
As stated above, infants with omphalocele who have
serious or life-threatening associated anomalies, especially
cardiac, should probably be treated non-operatively with
application of escharotic agents to the sac.
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2a,b Although some surgeons advocate leaving the sac

intact and repairing the fascia and skin over it, most
surgeons favor excision of the sac to allow complete intra-
abdominal exploration. The sac is sharply removed at the
2a
skin/fascia edge, with careful identification and ligation of
the umbilical vessels.

2b

3 The abdominal cavity can be enlarged by manual

stretching.

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 Operations  303

4 The skin is carefully ‘undermined’, separating it from

the deep fascia layers.

5 The fascia is closed with running or interrupted

absorbable sutures (polyglactin or polydioxanone) and the
umbilicus reconstructed.

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6a
Large omphalocele: staged repair
6a–c Large omphaloceles, frequently containing most of
the liver, are usually not fully reducible at the first operation,
and staged repair is necessary. After undermining the skin,
the skin is closed over the abdominal viscera, producing a
ventral hernia that can be repaired 6–12 months later.

6b

6c

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 Operations  305

7a,b An alternative approach utilizes prosthetic closure 7a

of the fascia defect over polyethylene or Silastic sheeting to
prevent adhesion of the viscera to the prosthetic material.
The skin is closed over the fascia prosthesis.

Polyethylene
or Silastic

Prosthesis

7b

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8a,b At 4–6-week intervals, the wound can be reopened

and the skin dissected from the prosthesis. The central 8a
portion of the prosthesis and sheeting are resected and
resutured to pull the fascia together. Eventually, the fascia
can be closed without the prosthesis.

8b

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 Operations  307

9a–d In cases of ruptured omphalocele, an alternative
method of management is necessary if the viscera cannot
be reduced primarily and there is insufficient skin for
coverage. A Dacron-reinforced Silastic sheet is attached
to the abdominal wall with a running, non-absorbable
suture and fashioned into a ‘pouch’ using the same suture.
A preformed ‘pouch’, with a spring-loaded base that fits
into the abdominal cavity to hold the ‘pouch’ in place and
therefore does not require suturing, is also commercially
available.
The viscera are gradually reduced into the abdominal
cavity, using gentle squeezing pressure on top of the
‘pouch’, which is then occluded by umbilical tape tie or
suture to maintain reduction. This is usually performed
without anesthesia every other day, over a 7–10-day
period, until the gut is fully reduced.

9a 9b 9c 9d

10 The infant is then returned to the operating room for

removal of the ‘pouch’, with fascia and/or skin closure.
A newer approach to infants with large omphalocele is
the use of progressive wrapping of the defect. By this
method, the amnion is left intact. The surgeon applies
an antibacterial ointment to the amnion, and then wraps
the defect first with gauze, and then an elastic wrap. By
gradually applying more pressure in downward and lateral
directions, the herniated organs gradually return to the
abdominal cavity, and the cavity, itself, grows in size.
While this process may take several months to fully reduce
the herniation, the gradual process often avoids respiratory
embarrassment, and greatly facilitates the eventual closure.
Great care must be take to avoid trauma to the amnion 10
early on, as excessive shear forces could tear the sac,
resulting in a significantly more complicated form of
reduction.

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Postoperative care
All infants should be maintained on systemic antibiotics for
5–7 days or until the prostheses are removed. Intravenous
nutrition should be initiated as soon as possible and
continued until bowel function returns. In infants with
omphalocele, this may be a period of several days.
Respiratory compromise is common after primary
repair, or during staged reduction of omphalocele, and
endotracheal intubation, ventilators, sedation, and,
occasionally, muscle relaxants are common interventions
in these infants in the early postoperative period.
Outcome
The outcome in these infants depends on the presence
and degree of prematurity, associated anomalies, and the
loss of bowel length due to atresia or gut infarction from
mesenteric vascular compromise.
Neonates with omphalocele have a high (50–60 percent)
incidence of associated anomalies and chromosome
abnormalities that precludes long-term survival in 20–30
percent of cases. Severe cardiac defects are particularly
troublesome in these infants. Modern neonatal intensive
care, improved ventilator management, and intravenous
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nutrition have undoubtedly been responsible for the
continued improvement in survival for these critically ill
infants.
Further reading
Christisan-Lagay ER, Kelleher CM, Langer JC. Neonatal
abdominal wall defects. Seminars in Fetal and
Neonatal Medicine 2011; 16: 164–72.
Grosfeld JL, Dawes L, Weber TR. Congenital abdominal
wall defects; current management and survival.
Surgical Clinics of North America 1981; 61: 1037–49.
Grosfeld JL, Weber TR. Congenital abdominal wall defects:
gastroschisis and omphalocele. Current Problems in
Surgery 1982; 19: 159–213.
Schuster SR. A new method for the staged repair of large
omphaloceles. Surgery, Gynecology and Obstetrics
1967; 125: 837–50.
Van Ejick FC, Aronson DA, Hoogeveen YL, Wijnen
RM. Past and current surgical treatment of giant
omphalocele. Journal of Pediatric Surgery 2011; 46:
482–8.
Weber TR, Au-Fliegner M, Downard C, Fishman S.
Abdominal wall defects. Current Opinion in Pediatrics
2002; 14: 491–7.
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